Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To identify the types of liver disease in which osteopenia is a prominent feature and to understand the mechanisms of bone loss, bone mineral density was measured in the lumbar spine and hip, bone alkaline phosphatase, osteocalcin, and biochemical markers of calcium homeostasis were measured in 42 women, aged 33 to 52, with chronic liver disease and in 299 healthy women of similar age. In control women, bone alkaline phosphatase and osteocalcin correlated negatively with bone density at all sites (p less than 0.05). In women with liver disease, osteocalcin correlated negatively with bone density in the lumbar spine (p less than 0.007), whereas bone alkaline phosphatase did not correlate with bone density at any site. Bone alkaline phosphatase correlated positively with osteocalcin in control women (p = 0.001) and negatively with osteocalcin in women with liver disease (p = 0.03). Serum bone alkaline phosphatase in women with liver disease was increased significantly over serum bone alkaline phosphatase of control women, probably because of decreased clearance owing to defective function or decreased numbers of hepatic asialoglycoprotein receptors. Bone density was lower in the lumbar spines and hips of women with primary sclerosing cholangitis, primary biliary cirrhosis, and chronic active hepatitis or fibrosis without cirrhosis than in the lumbar spine and hips of control women. However, the differences were not significant, possibly because of the small sample size. It is concluded that, in liver disease, osteocalcin is a more reliable marker of osteoblastic function than bone alkaline phosphatase. Although our results show that bone density may decrease in women with cholestatic liver disease, larger studies are needed to determine the degree of osteopenia.
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PMID:Osteocalcin and bone alkaline phosphatase in the serum of women with liver disease. 195 79

The plasma metallothionein concentration was evaluated in healthy subjects and in patients with several types of liver disorders. Plasma metallothionein concentrations in controls varied between 2.4 and 4.8 ng/ml. Patients with disorders associated with increased liver copper concentrations (i.e., primary biliary cirrhosis and primary sclerosing cholangitis) had significantly (both p less than 0.002) elevated plasma metallothionein concentrations (range = 1.8 to 52.2 ng/ml), and a considerable number of these were above the maximum control level (21 of 41 patients). In contrast, patients with liver disorders not associated with increased liver copper concentrations (alcoholic and cryptogenic cirrhosis, and acute viral and chronic active hepatitis) generally had normal plasma metallothionein concentrations and only a few were above the maximum control level (11 of 64 patients, maximum = 8.8 ng/ml). The metallothionein concentrations in plasma samples from patients in stage I or II primary biliary cirrhosis were within or slightly above the control range, whereas most patients in stage III had elevated levels (p less than 0.002), and almost all patients in stage IV had clearly elevated (p less than 0.0001) concentrations. In primary biliary cirrhosis the plasma metallothionein concentration tended to increase during the evolution of the disorder, and the concentration correlated significantly with the serum total bilirubin concentration. In conclusion, the plasma metallothionein concentration is significantly elevated in patients with primary biliary cirrhosis and in patients with primary sclerosing cholangitis. Although related to the histological stage of primary biliary cirrhosis, the measurement of plasma metallothionein concentrations contributes little to the diagnosis or the evaluation of the severity of these disorders.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Plasma metallothionein concentration in patients with liver disorders: special emphasis on the relation with primary biliary cirrhosis. 195 46

The natural history of primary sclerosing cholangitis (PSC) is poorly defined and its management remains controversial. Forty-eight symptomatic patients (median age 39 years, range 8-67 years; 30 male) with PSC were reviewed retrospectively. Thirty patients had inflammatory bowel disease. Four patients (8 per cent) developed or had an associated malignancy. Twenty-one (44 per cent) died; overall 5 year actuarial survival was 30 per cent. Twenty-three patients had 27 non-transplant related biliary operations (16 patients specifically for PSC) of whom 12 died. Serum bilirubin was the only parameter to improve after biliary surgery. Seventeen patients (35 per cent) underwent orthotopic liver transplantation (OLT) of whom nine are currently alive (1 year projected survival of 55 per cent). Previous biliary surgery correlated with a poor outcome (P less than 0.0001) after OLT. Being male, presence of cirrhosis, duration of symptomatic disease (greater than 3 years) and a serum bilirubin level greater than 100 mumol/l at presentation, were independently associated with a poor outcome (P less than 0.05). These data provide evidence that PSC is a progressive disease and conventional surgical options have little influence on the outcome. Previous biliary surgery adversely affects outcome following OLT. For progressive liver disease, liver transplantation should be considered the treatment of choice.
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PMID:Primary sclerosing cholangitis: surgical options, prognostic variables and outcome. 205 7

Twenty-one patients with primary sclerosing cholangitis were seen during 1979-87. The mean age at onset of disease was 51.7 years (range: 13-78 years) with a male: female ratio of 2.5:1. Six (29%) were asymptomatic at the time of diagnosis. Eleven patients (52%) had ulcerative colitis. Cholangiography demonstrated abnormalities limited to the intrahepatic ducts in 10 cases, with both intrahepatic and extrahepatic involvement in 11. Histological features on liver biopsy included: portal tract inflammation and cholestasis in all; paucity of bile ducts in 56%; piecemeal necrosis in 19% and cirrhosis in 6%. Circulating autoantibodies and elevated serum immunoglobulins were found in half of the patients and HLA-B8 was detected in 53%. A deficiency of circulating CD3 and CD8 cells was not found in the 12 patients tested. The mean follow-up was 51 months (range: 3-180 months). Three patients died from non-hepatic causes and another has received liver transplantation. A Kaplan-Meier curve predicted 70% survival at 72 months.
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PMID:Primary sclerosing cholangitis: clinical and immunopathological review of 21 cases. 210 94

By means of a case-report of a 33 years old pat., having colitis ulcerosa for 16 years with serious epitheldysplasia and developing primary sclerosing cholangitis, the typical progress of the disease with manifestation of a secondary biliary cirrhosis is shown. The course was complicated by the development of a bile-duct carcinoma. Present possibilities of diagnosis and therapy are discussed.
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PMID:[Metastatic bile duct cancer in secondary biliary cirrhosis associated with primary sclerosing cholangitis in ulcerative colitis]. 217 91

The study of chronic liver disease has been hampered by insufficient information relative to the pathogenesis of the many forms of hepatitis. Consequently, well-designed treatment strategies are frequently lacking. Wilson's disease is characterised by excessive copper accumulation in the liver and other organs. While d-penicillamine is clearly effective, many patients may not tolerate its many adverse effects. Trientine, oral zinc and unithiol have all shown promise as therapeutic alternatives. Autoimmune chronic active hepatitis responds well to prednisone and azathioprine. Cyclosporin has also produced clinical improvement in several case reports but no comparison has yet been made with the current standard therapy. Recombinant interferon-alpha (IFN alpha) has demonstrated the ability to inhibit hepatitis B viral replication, and the combination of oral corticosteroids followed by IFN alpha is more effective than either agent alone in eliminating viral replication in patients with chronic active hepatitis B. Currently, primary sclerosing cholangitis (PSC) has no standard medical management, but corticosteroids and methotrexate may each have a future role in its treatment. Drug treatment for primary biliary cirrhosis (PBC) has been disappointing, and early reports of success with d-penicillamine were not confirmed in large well-controlled trials. While some reports of improvement with several agents have been described, larger studies are still needed. Alcoholic liver disease continues to be associated with significant morbidity and mortality and numerous investigators have researched several different medical avenues of treatment. Success reported with androgens and the antithyroid agent propylthiouracil in alcoholic liver disease will need confirmation by other research before these agents can be recommended for routine use. Finally, colchicine may prove to be effective in slowing the rate of fibrosis in cirrhosis, but this has yet to be conclusively proven.
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PMID:Current therapy of chronic liver disease. 219 64

During a 12-year period, 46 children and adolescents with inflammatory bowel disease were followed from the time of diagnosis with regular biochemical tests of liver function. Thirty-four patients had ulcerative colitis and 12 had Crohn's disease. Mean age at the time of diagnosis was 10.2 years (range 7 months-17 years) and the mean follow-up period was 5.2 years (range 1-11 years). Pathological liver function tests were found in 60% of the 34 patients with ulcerative colitis: 9 of these 20 patients demonstrated more severe disturbance, usually at the time of diagnosis. Liver damage was most frequent in patients with total colitis. Liver biopsy was performed in eight patients, demonstrating "pericholangitis", fibrosis and in one case cirrhosis. Morphometry of electron microscopical pictures revealed a significantly increased number of lysosomes and dilated cisternae of the rough endoplasmic reticulum. ERCP was performed in two patients, verifying primary sclerosing cholangitis in one. Four of the 12 patients with Crohn's disease had mildly pathological liver function tests. No correlation was found to the extent, duration or treatment of bowel disease. In our series of juvenile inflammatory bowel disease, liver damage occurred frequently, especially in ulcerative colitis. The more severe changes tended to coincide with the onset of bowel disease.
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PMID:Liver damage in juvenile inflammatory bowel disease. 221 95

Primary sclerosing cholangitis is a rare disease of unknown etiology. Sclerosis of the bile ducts may actually be the final result of multiple factors such as autoimmune, bacterial, congenital, drug, or viral injury. The most commonly associated diseases are ulcerative colitis and chronic pancreatitis. Except in the earliest stages of the disease, liver histologic findings are not specific. Most patients present with jaundice, pain, and pruritus, although an increasing number of asymptomatic patients with inflammatory bowel disease and abnormal liver function are being identified. Cholangiography is key to the diagnosis and is usually pathognomonic except in the unusual case where primary sclerosing cholangitis is confused with cholangiocarcinoma. Many forms of medical therapy have been tried, including antibiotics, azathioprine, cholestyramine, colchicine, cyclosporine, D-penicillamine, steroids, and ursodeoxycholic acid. To date, none of these medications has been proved to alter the course of this disease. Recent reports of ursodeoxycholic acid trials have been encouraging, but long-term results of ongoing randomized trials have yet to be published. In recent years, balloon dilatation of biliary strictures has been accomplished via endoscopic and percutaneous transhepatic approaches. However, in patients with primary sclerosing cholangitis, these nonoperative manipulations must be done repeatedly, may entail multiple general anesthetics, and are difficult to perform. We believe that a direct surgical approach to the biliary tree with long-term transhepatic stenting is indicated in selected patients with severe hilar or extrahepatic stricturing, persistent jaundice or recurrent cholangitis, and no evidence of cirrhosis. Hepatic transplantation should be reserved for patients with primary sclerosing cholangitis who have well-established cirrhosis and have not responded to other therapeutic measures.
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PMID:Primary sclerosing cholangitis. 224 21

The authors review the indications and outcome of liver transplantation for hepatic cirrhosis of various aetiologies in adults, on the basis of their experience and that of the literature. Up to 1989, they performed 107 liver transplantations in 93 adults, including 70 patients with cirrhosis: primary biliary cirrhosis (PBC) in 20, primary sclerosing cholangitis (PSC) in 2, secondary biliary cirrhosis in 1, post-necrotic cirrhosis in 35, alcoholic cirrhosis in 5, metabolic cirrhosis in 7. The best indications are PBC with rising bilirubinemia (greater than 5 mg/dl) or portal hypertension and PSC with severe and diffuse lesions. The outcome of transplantation was satisfactory in patients with PBC: 16 survived with a mean follow-up of 23 months. In patients transplanted for postnecrotic cirrhosis the outcome might be better if HBS is associated preoperatively with delta antigen: 6 patients of this group survived with a mean follow-up of 12.6 months while in the group of 8 patients with HBS not associated preoperatively with delta antigen, 6 patients survived with a mean follow-up of 12.8 months. There was a trend toward a higher hospital mortality after transplantation in cirrhotic patients with pulmonary arterio-venous shunts in comparison with cirrhotic patients without significant pulmonary arterio-venous shunts preoperatively.
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PMID:[Hepatic transplantation in cirrhosis]. 226 7

Usually, ascending cholangitis is a bacterial process. However, in the debilitated or immunocompromised patient, mycotic cholangitis must be placed in the differential diagnosis. We report a patient with cryptogenic cirrhosis whose presenting problem in his terminal hospitalization was spontaneous bacterial peritonitis, for which he was treated with broad-spectrum antibiotics. Endoscopic retrograde cholangiopancreatogram was performed during the hospital course to explain his profound hyperbilirubinemia. The findings were grossly consistent with primary sclerosing cholangitis or cholangiocarcinoma. The patient subsequently continued to deteriorate, and died with hepatic and renal failure. At autopsy, he was found to have choledocholithiasis, marked biliary duct proliferation, and ascending cholangitis, with Trichosporon demonstrated histologically to be invading the bile ducts. To our knowledge, this is the first reported case of Trichosporon cholangitis.
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PMID:Trichosporon cholangitis associated with hyperbilirubinemia, and findings suggesting primary sclerosing cholangitis on endoscopic retrograde cholangiopancreatography. 229 69


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